LEARN CENTER

TYPES OF SCLERODERMA: LOCALIZED

Localized scleroderma usually only affects a few places on the skin or muscles.

The scleroderma rarely spreads elsewhere, and internal organs are typically unaffected. Generally, localized scleroderma is relatively mild. Although localized scleroderma is not fatal, the condition may still adversely affect quality of life. Patients with localized scleroderma rarely develop the systemic form. 

The four main types of localized scleroderma are morphea, generalized morphea, linear scleroderma, and en coup de sabre.
Morphea, characterized by waxy patches on the skin, does not affect internal organs.

The skin under these waxy patches may thicken. In addition, the fatty layer underneath the morphea spots is usually lost. The patches, which tend to be lighter or darker than the surrounding skin and thus tend to stand out, can vary in size from half an inch to six inches or more in diameter. Morphea patches can enlarge, shrink or even disappear spontaneously. Although morphea usually appears between the ages of 20 and 50, it is often seen in young children.

Linear scleroderma frequently begins as a streak or line of hardened, waxy skin on the patient’s arm, leg, or forehead.

Sometimes, a long crease on the head or neck forms; this crease is referred to as “en coup de sabre” because of the resemblance to a saber (sword) wound. Linear scleroderma typically involves the deeper skin layers as well as the surface layers. Sometimes, the motion of the joints that lie underneath the skin is also affected. The fatty layer under the skin can be lost, resulting in an affected limb that is thinner than the other one. In growing children, linear scleroderma may cause the limb to be shorter than the unaffected one. Linear scleroderma typically develops during childhood.